Shannon van Hoorn

40 Chapter 2 Author, (year), country Study design and characteristics of population (n; gender; disease type; age) PROMs Measured PROs PROs and conclusions* de Wee, E. M., et al. (2010). The Netherlands 25 Prospective, multicenter 509 patients with VWD (62% female) • Type 1: 282 • Type 2: 196 • Type 3: 21 • Unspecified: 10 Age (years): • range: 16-87 SF-36 • HRQoL • Physical activity • General health • Pain • Women have lower HRQoL compared to normative data, especially on the domains of general health, vitality and physical component summary (p<0.001). • Men have a lower HRQoL compared to normative data, especially on the domains pain and vitality (p<0.005). • Higher bleeding scores have a negative impact on physical domains of HRQoL (p<0.001). • Patients with VWD type 3 score lower in the domains physical functioning, pain and physical component summary, compared to patients with VWD type 1 and 2 (p<0.05). de Wee, E. M., et al. (2011). The Netherlands 24 Prospective, multicenter 133 patients with VWD (41% females) • Type 1: 69 • Type 2: 49 • Type 3: 15 Age (years) • range: 0.3-17 CHQ-CF87 CHQ-PF50 ITQOL • HRQoL • Physical functioning • General health • Psychosocial functioning • Pain • Preschool children have lower HRQoL scores compared to normative data, especially in the domains of general health and parental time impact (p≤0.05) and higher scores in the domain change in health (p≤0.05). • Schoolchildren have lower HRQoL compared to normative data, especially in the domains of physical functioning, role functioning – emotional/behavioral, general health and physical summary (p≤0.05). • There is no difference in HRQoL between boys and girls (preschool and schoolchildren). • Schoolchildren with VWD type 3 have lower HRQoL compared to patients with VWD type 1 and 2 and normative data, especially in the domains of pain, general health, parental impactemotional, family activities and physical summary (p≤0.05). • Schoolchildren with severe bleeding phenotype have lower HRQoL scores in the social, emotional and physical domains (p≤0.05).

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